Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patients
Autor(a) principal: | |
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Data de Publicação: | 2006 |
Outros Autores: | , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Revista Brasileira de Reumatologia (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042006000500002 |
Resumo: | INTRODUCTION: cryoglobulinemia vasculitis (CV) is a systemic vasculitis secondary to circulating immune complex deposition in small blood vessels. In the overwhelming majority of patients, it is associated to hepatitis C virus (HCV) infection, nevertheless it has been observed in a wide variety of diseases and in patients with no underling conditions - "essential cryoglobulinemia". OBJECTIVE: to report a series of patients with non-HCV CV. PATIENTS AND METHODS: a retrospective chart review of non-HCV CV patients was carried out by members of the Minas Gerais Vasculitis Study Group. Demographics, clinical, laboratory data, treatments and follow-up are reported. RESULTS: nine patients, two men and seven women, aged a 55.3 ± 17 years were studied. In three patients, CV was attributed to connective tissue disease and in one to a monoclonal gammopathy of undetermined significance (MGUS). Five patients presented essential cryoglobulinemia. Four patients presented bone marrow lymphoid infiltrates, but none developed overt lymphoma. All patients complained of myalgia/arthralgia. Eight patients (89%) presented cutaneous vasculitis; four patients (44%) had glomerulonephritis (GN) and six patients (67%) had neuropathy. Regarding treatment, six patients responded to steroids and immunosuppressive agents. Three patients were refractory to conventional therapy and therefore were treated with rituximab. After a follow-up of 19.3 ± 14 months, five patients (56%) were in partial remission and four patients (44%) were in complete remission. CONCLUSION: non-HCV cryoglobulinemia may present itself as a systemic vasculitis. It should not be understood as a simple inflammatory disease, since most patients present evidence of a lymphoproliferative disorders. Treatment should include steroids and immunosuppressive agents. Refractory patients may benefit themselves from rituximab. |
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Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patientssystemic vasculitiscryoglobulinemiacryoglobulinemic vasculitisINTRODUCTION: cryoglobulinemia vasculitis (CV) is a systemic vasculitis secondary to circulating immune complex deposition in small blood vessels. In the overwhelming majority of patients, it is associated to hepatitis C virus (HCV) infection, nevertheless it has been observed in a wide variety of diseases and in patients with no underling conditions - "essential cryoglobulinemia". OBJECTIVE: to report a series of patients with non-HCV CV. PATIENTS AND METHODS: a retrospective chart review of non-HCV CV patients was carried out by members of the Minas Gerais Vasculitis Study Group. Demographics, clinical, laboratory data, treatments and follow-up are reported. RESULTS: nine patients, two men and seven women, aged a 55.3 ± 17 years were studied. In three patients, CV was attributed to connective tissue disease and in one to a monoclonal gammopathy of undetermined significance (MGUS). Five patients presented essential cryoglobulinemia. Four patients presented bone marrow lymphoid infiltrates, but none developed overt lymphoma. All patients complained of myalgia/arthralgia. Eight patients (89%) presented cutaneous vasculitis; four patients (44%) had glomerulonephritis (GN) and six patients (67%) had neuropathy. Regarding treatment, six patients responded to steroids and immunosuppressive agents. Three patients were refractory to conventional therapy and therefore were treated with rituximab. After a follow-up of 19.3 ± 14 months, five patients (56%) were in partial remission and four patients (44%) were in complete remission. CONCLUSION: non-HCV cryoglobulinemia may present itself as a systemic vasculitis. It should not be understood as a simple inflammatory disease, since most patients present evidence of a lymphoproliferative disorders. Treatment should include steroids and immunosuppressive agents. Refractory patients may benefit themselves from rituximab.Sociedade Brasileira de Reumatologia2006-10-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042006000500002Revista Brasileira de Reumatologia v.46 n.5 2006reponame:Revista Brasileira de Reumatologia (Online)instname:Sociedade Brasileira de Reumatologia (SBR)instacron:SBR10.1590/S0482-50042006000500002info:eu-repo/semantics/openAccessCruz,Boris A.Melo,Ana Lúcia V. deCruz Filho,Achiles de AlmeidaGouvea,Patrícia SaloméSoares,César José G.Batlle,Viviane S.Hybner,Maria Luiza T.Goulart,Fabiana B.Pádua,Paulo M. deeng2006-12-05T00:00:00Zoai:scielo:S0482-50042006000500002Revistahttp://www.scielo.br/scielo.php?script=sci_serial&pid=0482-5004&lng=pt&nrm=isoONGhttps://old.scielo.br/oai/scielo-oai.php||sbre@terra.com.br1809-45700482-5004opendoar:2006-12-05T00:00Revista Brasileira de Reumatologia (Online) - Sociedade Brasileira de Reumatologia (SBR)false |
dc.title.none.fl_str_mv |
Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patients |
title |
Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patients |
spellingShingle |
Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patients Cruz,Boris A. systemic vasculitis cryoglobulinemia cryoglobulinemic vasculitis |
title_short |
Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patients |
title_full |
Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patients |
title_fullStr |
Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patients |
title_full_unstemmed |
Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patients |
title_sort |
Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patients |
author |
Cruz,Boris A. |
author_facet |
Cruz,Boris A. Melo,Ana Lúcia V. de Cruz Filho,Achiles de Almeida Gouvea,Patrícia Salomé Soares,César José G. Batlle,Viviane S. Hybner,Maria Luiza T. Goulart,Fabiana B. Pádua,Paulo M. de |
author_role |
author |
author2 |
Melo,Ana Lúcia V. de Cruz Filho,Achiles de Almeida Gouvea,Patrícia Salomé Soares,César José G. Batlle,Viviane S. Hybner,Maria Luiza T. Goulart,Fabiana B. Pádua,Paulo M. de |
author2_role |
author author author author author author author author |
dc.contributor.author.fl_str_mv |
Cruz,Boris A. Melo,Ana Lúcia V. de Cruz Filho,Achiles de Almeida Gouvea,Patrícia Salomé Soares,César José G. Batlle,Viviane S. Hybner,Maria Luiza T. Goulart,Fabiana B. Pádua,Paulo M. de |
dc.subject.por.fl_str_mv |
systemic vasculitis cryoglobulinemia cryoglobulinemic vasculitis |
topic |
systemic vasculitis cryoglobulinemia cryoglobulinemic vasculitis |
description |
INTRODUCTION: cryoglobulinemia vasculitis (CV) is a systemic vasculitis secondary to circulating immune complex deposition in small blood vessels. In the overwhelming majority of patients, it is associated to hepatitis C virus (HCV) infection, nevertheless it has been observed in a wide variety of diseases and in patients with no underling conditions - "essential cryoglobulinemia". OBJECTIVE: to report a series of patients with non-HCV CV. PATIENTS AND METHODS: a retrospective chart review of non-HCV CV patients was carried out by members of the Minas Gerais Vasculitis Study Group. Demographics, clinical, laboratory data, treatments and follow-up are reported. RESULTS: nine patients, two men and seven women, aged a 55.3 ± 17 years were studied. In three patients, CV was attributed to connective tissue disease and in one to a monoclonal gammopathy of undetermined significance (MGUS). Five patients presented essential cryoglobulinemia. Four patients presented bone marrow lymphoid infiltrates, but none developed overt lymphoma. All patients complained of myalgia/arthralgia. Eight patients (89%) presented cutaneous vasculitis; four patients (44%) had glomerulonephritis (GN) and six patients (67%) had neuropathy. Regarding treatment, six patients responded to steroids and immunosuppressive agents. Three patients were refractory to conventional therapy and therefore were treated with rituximab. After a follow-up of 19.3 ± 14 months, five patients (56%) were in partial remission and four patients (44%) were in complete remission. CONCLUSION: non-HCV cryoglobulinemia may present itself as a systemic vasculitis. It should not be understood as a simple inflammatory disease, since most patients present evidence of a lymphoproliferative disorders. Treatment should include steroids and immunosuppressive agents. Refractory patients may benefit themselves from rituximab. |
publishDate |
2006 |
dc.date.none.fl_str_mv |
2006-10-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042006000500002 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042006000500002 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/S0482-50042006000500002 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Sociedade Brasileira de Reumatologia |
publisher.none.fl_str_mv |
Sociedade Brasileira de Reumatologia |
dc.source.none.fl_str_mv |
Revista Brasileira de Reumatologia v.46 n.5 2006 reponame:Revista Brasileira de Reumatologia (Online) instname:Sociedade Brasileira de Reumatologia (SBR) instacron:SBR |
instname_str |
Sociedade Brasileira de Reumatologia (SBR) |
instacron_str |
SBR |
institution |
SBR |
reponame_str |
Revista Brasileira de Reumatologia (Online) |
collection |
Revista Brasileira de Reumatologia (Online) |
repository.name.fl_str_mv |
Revista Brasileira de Reumatologia (Online) - Sociedade Brasileira de Reumatologia (SBR) |
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